Abstract:
BACKGROUND:Patients with autoimmune lymphoproliferative syndrome (ALPS) have an autosomal dominant genetic defect that affects lymphocyte apoptosis and is associated with chronic nonmalignant lymphadenopathy, splenomegaly, and autoimmunity, particularly affecting RBCs, WBCs, and platelets. STUDY DESIGN AND METHODS:DATs were performed on 34 consecutive patients with ALPS and 37 of their clinically unaffected relatives. The effects of age, sex, race, and immunoglobulin levels on the incidence of autoantibodies and clinical hemolysis were assessed. RESULTS:The DAT was positive in 21 (62%) of ALPS patients but in only 1 (3%) of their relatives (p = 0.001). The DAT reacted because of IgG alone in 43 percent, complement alone in 5 percent, and IgG plus complement in 19 percent; 33 percent of the patients' cells had a positive reaction with polyspecific reagent only. All 10 ALPS patients with a history of hemolytic anemia had a positive DAT. Sixty percent of them had only IgG on their cells, 30 percent had IgG and complement, and 10 percent reacted only with polyspecific reagent. Of the 11 patients with a positive DAT and no history of hemolytic anemia, IgG alone was present in 27 percent, complement alone in 9%, and IgG plus complement in 9 percent; 55 percent had positive DATs only with polyspecific reagent. Among ALPS patients, those with a positive DAT had greater quantities of cells with increased alpha and ss T-cell receptors that phenotyped as CD4-CD8- and higher IgG levels. CONCLUSIONS:The DAT results in ALPS patients are most similar to those found in warm autoimmune hemolytic anemia. The DAT is useful to distinguish affected and unaffected persons within an ALPS family.
journal_name
Transfusionjournal_title
Transfusionauthors
Stroncek DF,Carter LB,Procter JL,Dale JK,Straus SEdoi
10.1046/j.1537-2995.2001.41010018.xsubject
Has Abstractpub_date
2001-01-01 00:00:00pages
18-23issue
1eissn
0041-1132issn
1537-2995journal_volume
41pub_type
杂志文章相关文献
TRANSFUSION文献大全abstract::The ability of anti-D (Rho) reagents containing chemically modified and unmodified IgG molecules to detect fetal-maternal hemorrhages was compared using mixtures of Rh negative adult red blood cells and Rh positive cord red blood cells. No significant differences among reagents were observed in this small study. These...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1982.22182154226.x
更新日期:1982-01-01 00:00:00
abstract::Tunneled central venous catheters with ports are increasingly used for therapeutic apheresis procedures. Vortex ports have been used as access for therapeutic apheresis procedures, but are not ideal for therapeutic plasma exchange (TPE) procedures due to lower flow rates. We performed an in vitro experiment to compare...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14494
更新日期:2018-02-01 00:00:00
abstract:BACKGROUND:In August 2016, the Food and Drug Administration advised US blood centers to screen all whole blood and apheresis donations for Zika virus (ZIKV) with an individual-donor nucleic acid test (ID-NAT) or to use approved pathogen reduction technology (PRT). The cost of implementing this guidance nationally has n...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14164
更新日期:2017-06-01 00:00:00
abstract::A microwave oven has been specifically modified to permit rapid thawing of fresh-frozen plasma (FFP) by using a rotating disc with a temperature sensor to hold the plasma bag. This modification makes it possible to mix the FFP continuously during thawing, and automatically shuts the oven off when the plasma reaches 21...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1984.24184122564.x
更新日期:1984-01-01 00:00:00
abstract:BACKGROUND:Used leuko-depletion filters (LDFs), containing billions of white blood cells (WBCs), are discarded. Because the steady-state blood contains low quantities of stem and progenitor cells that are retained in LDFs, the viability and the functional properties of mononuclear cells (MNCs) and CD 34+ cells recovere...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2005.00677.x
更新日期:2006-01-01 00:00:00
abstract:BACKGROUND:Hypothermic storage transforms red blood cells (RBC) from smooth biconcave discocytes into increasingly spherical spiculated echinocytes and, ultimately, fragile spherocytes (S). Individual cells undergo this transformation at different rates, producing a heterogeneous mixture of RBCs at all stages of echino...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.15979
更新日期:2020-08-04 00:00:00
abstract:BACKGROUND:Variant Creutzfeldt-Jakob disease (vCJD) has been transmitted by blood transfusion (TTvCJD). The US Food and Drug Administration (FDA) recommends deferring blood donors who resided in or traveled to 30 European countries where they may have been exposed to bovine spongiform encephalopathy (BSE) through beef ...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.13971
更新日期:2017-04-01 00:00:00
abstract:BACKGROUND:A new citrate-acetate-NaCl platelet additive solution, identified as PAS 2, was developed to prepare platelet concentrates (PCs) from pooled 0.5 CPD buffy coats (BCs). STUDY DESIGN AND METHODS:A study was undertaken to evaluate PAS 2 in vitro (n = 8) and in vivo (n = 9) against a commercially available solu...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1995.35195090662.x
更新日期:1995-01-01 00:00:00
abstract:BACKGROUND:Concerns have been raised that aging of the general population will increase the demand for blood products. Modeling can be applied to assess trends in blood demand and supply and predict how these will develop over time. STUDY DESIGN AND METHODS:We developed mathematical models to describe and predict the ...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2010.02716.x
更新日期:2010-11-01 00:00:00
abstract:BACKGROUND:Tests for platelet-specific antibodies are important in the diagnosis of immune platelet disorders. Monoclonal antibody glycoprotein capture assays have been the gold standards in platelet antibody detection for almost 30 years. However, such assays are complex and cumbersome to perform, which limits their r...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14122
更新日期:2017-07-01 00:00:00
abstract:BACKGROUND:Recently, a more accurate and reliable screening test has been investigated for noninvasive prenatal fetal RHD genotyping from D- women. The objective of this study was to perform the new method of noninvasive fetal RHD genotyping with maternal plasma from D- women by use of capillary electrophoresis. STUDY...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2008.01681.x
更新日期:2008-06-01 00:00:00
abstract:BACKGROUND:The von Willebrand factor (VWF)-cleaving protease, ADAMTS13, is often deficient in cases of thrombotic thrombocytopenic purpura (TTP). The primary treatment of TTP is therapeutic plasma exchange (TPE) utilizing a variety of plasma products that help restore ADAMTS13 activity. However, multiple replacement pr...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2007.01074.x
更新日期:2007-01-01 00:00:00
abstract::Although the high (40 to 50 per cent) glycerol method of freezing red blood cells has many advantages, no feasible procedure for deglycerolizing with only a clinical centrifuge has been available. Thus this method has been restricted to blood centers that distribute a sufficient number of frozen cells to warrant the i...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1977.17578014580.x
更新日期:1977-09-01 00:00:00
abstract::Donor deferral rates in regional blood centers vary from 5 to 24 per cent, reducing by more than 1,250,000 the number of units of volunteer blood available for transfusion in the nation each year. Those criteria for donor deferral which are intended to exclude donors likely to suffer a "donor reaction" are based parti...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1980.20581034503.x
更新日期:1980-09-01 00:00:00
abstract:BACKGROUND:The RhD blood group antigen is extremely polymorphic and the DEL phenotype represents one such class of polymorphisms. The DEL phenotype prevalent in East Asian populations arises from a synonymous substitution defined as RHD*1227A. However, initially, based on genomic and cDNA studies, the genetic basis for...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14439
更新日期:2018-03-01 00:00:00
abstract:BACKGROUND:Transfusion-transmitted babesiosis (TTB) has been rapidly increasing in incidence since the beginning of the 21st century. Asymptomatic individuals with Babesia infection are able to donate blood in the United States because of the lack of specific blood donation testing. Blood products collected in Babesia-...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14246
更新日期:2017-10-01 00:00:00
abstract::In spite of growing awareness of the potential risks associated with transfusion, the number of platelet units transfused in the United States continues to increase each year. There is a growing interest in ensuring that all transfusions are administered for appropriate reasons. Prospective review of requests for tran...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1991.31791368335.x
更新日期:1991-09-01 00:00:00
abstract:BACKGROUND:Storage of blood units (for 35-42 days, depending on the preservative solution) has been reported to induce changes (e.g., reduction of sialic acid level) in red cells that are expected to alter their aggregability. STUDY DESIGN AND METHODS:The aggregability of stored red cells was monitored in their autolo...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1999.39399219284.x
更新日期:1999-03-01 00:00:00
abstract:BACKGROUND:Variant RHCE alleles with diminished expression of C, c, E, and e antigens have been described and indicate the genetic diversity of this gene locus in several populations. In this study the molecular background of variant RhCE antigens identified by standard serologic routine testing in German blood donors ...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2009.02220.x
更新日期:2009-09-01 00:00:00
abstract:BACKGROUND:There are no reports in the transfusion medicine literature that describe adverse reactions and donor arm injuries after whole-blood donation based on solicited information. STUDY DESIGN AND METHODS:The present study solicited adverse reaction and donor arm injury information from 1000 randomly selected who...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.2003.00368.x
更新日期:2003-05-01 00:00:00
abstract:BACKGROUND:The ABO blood group is a major determinant in living donor kidney transplantation since AB antigens are expressed on renal tissue. Little attention has been directed to the ABH-secretor status of the donor kidney. As renal tissue is capable of secreting soluble ABH antigens in secretors, we examined the infl...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.13711
更新日期:2016-09-01 00:00:00
abstract:BACKGROUND:We assessed donor return rates, donation frequency, and factors related to the evolution of ferritin levels 2 years after entering donors into a large operational study of ferritin testing. STUDY DESIGN AND METHODS:Ferritin testing was done on donors from representative clinics (n = 12,595). Low-ferritin do...
journal_title:Transfusion
pub_type: 杂志文章,多中心研究,随机对照试验
doi:10.1111/trf.14941
更新日期:2018-12-01 00:00:00
abstract:BACKGROUND:Uncommonly, antibodies that appear to exhibit antigenic specificity on red blood cell (RBC) panels fail to maintain specificity following alloadsorption (i.e., they mimic antigenic specificity). Understanding both the pitfalls and the proper pathways to establish the diagnosis and to interpret the clinical s...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.0041-1132.2004.04112.x
更新日期:2004-09-01 00:00:00
abstract::Many strains of Escherichia coli possess an antigen that reacts with naturally occurring antibodies to human red cell B antigens. The authors tested the hypothesis that the presence of naturally occurring anti-B isoagglutinins afford protection against the development of E. coli septicemia. The blood groups found in 1...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1986.26687043620.x
更新日期:1986-11-01 00:00:00
abstract:BACKGROUND:Historically, paid blood donors were found to transmit hepatitis at higher rates than volunteers. In those older studies, paid donors frequently were recruited from prisons or slum areas--a finding consistent with the belief that monetary payment in itself did not necessarily lead to the high-risk status of ...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1994.34294143937.x
更新日期:1994-02-01 00:00:00
abstract:BACKGROUND:Recent blood shortages and the potential clinical impact of red blood cell (RBC) age highlight the need to understand blood supply delivery. This study addresses the characteristics and mechanics of RBC unit trafficking and storage across the transfusion service, previously undescribed in the literature. ST...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2009.02375.x
更新日期:2010-01-01 00:00:00
abstract::Lyophilized factor VIII concentrates, because of their convenience, are the most commonly used products for providing antihemophilic factor to patients with hemophilia A. Heightened concerns about associated complications indicate that cryoprecipitate usage will increase in the immediate future. We present a protocol ...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1983.23383224913.x
更新日期:1983-05-01 00:00:00
abstract::Many significant events have occurred in the recent past that beg a broad audience to address the question "What is pediatric transfusion medicine?" Herein, we list some of these events and their relevance below and attempt to provide an answer for this question. Indeed, several issues regarding the subspecialty of pe...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/j.1537-2995.2008.02015.x
更新日期:2009-03-01 00:00:00
abstract:BACKGROUND:Biological and technical variability has been increasingly appreciated as a key factor impacting red blood cell (RBC) storability and, potentially, transfusion outcomes. Here, we performed metabolomics analyses to investigate the impact of factors other than storage duration on the metabolic phenotypes of st...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1111/trf.14979
更新日期:2019-01-01 00:00:00
abstract:BACKGROUND:Methods that detect a child's homozygosity by examination of allelic products are insensitive for diagnosing incest because, at a given locus, a homozygous state is expected with a frequency of only 0.25 when parents are first-degree relatives. Furthermore, these methods are not specific if the population co...
journal_title:Transfusion
pub_type: 杂志文章
doi:10.1046/j.1537-2995.1994.34294143949.x
更新日期:1994-02-01 00:00:00